Medical treatment of systemic idiopathic fibrosis is first based on steroid therapy, of which efficacy is well known for nearly 75% of cases of retroperitoneal fibrosis, but les certain for the other localisations of the disease. Studies on the immuno-pathological mechanism of systemic fibrosis have led to immunosuppressive drug use, such as azathioprin, cyclophosphamide and methotrexate. Tamoxifen has been tried because of its efficacy on desmoid tumors and seems to be of great interest. Attention is focused on fibrosing cervicitis which may also be treated with immunosuppressive drugs.
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http://dx.doi.org/10.1016/s0248-8663(97)80116-6 | DOI Listing |
Rheumatology (Oxford)
January 2025
Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.
Objectives: To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.
Methods: A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry.
J Thorac Dis
December 2024
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Background: The benefits of pulmonary rehabilitation (PR) for patients with idiopathic pulmonary fibrosis (IPF) have been limited to improving dyspnea, exercise capacity, and quality of life (QoL). This study aimed to assess the current status of PR and its effect on prognosis.
Methods: The Nationwide Korean Health Insurance Review and Assessment Service (HIRA) database was used in this study.
Ther Adv Rare Dis
January 2025
Jinnah Medical and Dental College, Karachi, Pakistan.
Behçet's disease is a kind of variable vessel vasculitis (VVV) and inflammatory systematic disease affecting various organs of the body. The cause of the disease is idiopathic but is most commonly genetic in origin. A positive skin prick test (dermatographia), genital sores, eye irritation, skin sores, and at least three episodes of mouth sores in a year confirm the diagnosis.
View Article and Find Full Text PDFJ Dermatol Sci
January 2025
Biosciences Department, Durham University, Durham, United Kingdom. Electronic address:
Background: Systemic Sclerosis (SSc) is an idiopathic rheumatic inflammatory disease that is characterised by inflammation and skin fibrosis. Type I interferon is significantly elevated in the disease.
Objective: The objective of this study is to determine the role of the TCA cycle metabolite fumarate in SSc.
Mol Biol Rep
January 2025
Pediatric Rheumatology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.
Background: Interleukin-1 receptor-associated kinase1 (IRAK1) plays a considerable role in the inflammatory signaling pathway. The current study aimed to identify any association between (rs1059703) single nucleotide polymorphism (SNP) and vulnerability to rheumatological diseases in the pediatric and adult Egyptian population.
Patients And Methods: The current study included four patient groups: adult Systemic lupus erythematosus (SLE), Rheumatoid arthritis (RA), juvenile systemic lupus erythematosus (JSLE), and juvenile idiopathic arthritis (JIA).
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